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货号： bs-9068R-Cy3 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-9068R-Cy3
- 英文名称
- Anti-HMBS/Cy3
- 中文名称
- Cy3标记的卟胆原脱氨酶抗体
- 别 名
- HEM3_HUMAN; HMBS; Hydroxymethylbilane synthase; PBG D; PBG-D; PBGD; Porphobilinogen deaminase; Pre uroporphyrinogen synthase; Pre-uroporphyrinogen synthase; UPS; Uroporphyrinogen I synthase; Uroporphyrinogen I synthetase.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 免疫学 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Pig, Cow, Rabbit, Sheep, Cat,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 39kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human HMBS
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
Function:
Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
Subcellular Location:
Cytoplasm.
Tissue Specificity:
Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
DISEASE:
Defects in HMBS are the cause of acute intermittent porphyria (AIP) [MIM:176000]. AIP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.
Similarity:
Belongs to the HMBS family.
Database links:Entrez Gene: 3145Human
Entrez Gene: 15288Mouse
Entrez Gene: 396581Pig
Entrez Gene: 25709Rat
Omim: 609806Human
SwissProt: P08397Human
SwissProt: P22907Mouse
SwissProt: P19356Rat
Unigene: 82609Human
Unigene: 247676Mouse
Unigene: 11080Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
卟胆原脱氨酶，为血红素等四吡咯环化合物合成通路中的催化酶。PBGD催化卟胆原(Porphobilingen，PBG)底物的线性四聚化，反应过程中四分子卟胆原底物有序性的共价连接到酶的辅基上，最后水解形成具有四联吡咯环结构的尿卟啉原前体。PBGD的活力缺陷会引起急性间歇性卟啉症(Acute Intermittant Porphyria，AIP)的发生。